Inflammatory myofibroblastic tumor with CNS involvement

Abstract

Background: Inflammatory myofibroblastic tumors (IMT) represent a spectrum of neoplasms that occur in the mesentery and retroperitoneum and less frequently in the mediastinum of children and young adults. Transformation into inflammatory fibrosarcoma and metastases are rare.

Case report: We report the case of a 16-year-old patient with an inflammatory myofibroblastic tumor of the mesentery with mediastinal metastases. Partial remission was obtained by chemotherapy with ifosfamide, dactinomycine, and vincristine. Two months later, relapse with infiltration of the meninges developed, and the patient died.

Conclusion: This case demonstrates unusual features of an IMT: presentation with metastases, excellent response to chemotherapy, dissemination to the CNS.